i t i t pip arasitic twins - suny downstate medical...

P iti T iParasitic Twins

SUNY Downstate Medical CenterSUNY Downstate Medical CenterPediatric Surgery

J i K MDJamie Kang, MD

www.downstatesurgery.org

CaseCase

A full-term baby girl born to a xx yo healthy female via c-y g y ysectionMaternal history: - P2042- HIV/ Hep B/ VDRL negative, Rubella immuned

prenatal sonogram showed possible meningocele and- prenatal sonogram showed possible meningocele and left lower extremity agenesis

- amniocentesis analysis: 46: XX: gene deletion at locusamniocentesis analysis: 46: XX: gene deletion at locus22 of X chromosome

CaseCase

Apgars score: 1min – 4, 5 min – 6, 10min – 7pg , ,No spontaneous breathingHR < 100, sat 50%A large defect and a soft tissue mass noted at left lower quadrantImperforate anusImperforate anus2 vessels cord

CaseCasewww.downstatesurgery.org

Pre-op Differential DiagnosesPre op Differential Diagnoses

Cloacal exstrophy MeningomyeloceleMeningomyeloceleIncomplete conjoined twins

OperationOperation

Part I: Excision of meningocele

Part II: Exploratory laparotomy

Part III: GU system identification

Part IV: Excision of malformed tissue

Part V: End colostomy creation

Part II: Exploratory Laparotomy

Part II: Exploratory laparotomyPart II: Exploratory laparotomywww.downstatesurgery.org

Part III: GU system identification

Part III: GU system identificationPart III: GU system identificationwww.downstatesurgery.org

Part IV: Excision of the malformed tissuePart IV: Excision of the malformed tissuewww.downstatesurgery.org

Part V: Creation of colostomyPart V: Creation of colostomywww.downstatesurgery.org

PathologyPathology

Tissue source Final Pathology

Unknown foreign tissue Fragments of skin (scalp), bone and glial tissue

r/o ectopic kidney tissue Fragments of glial vascular tissuer/o ectopic kidney tissue Fragments of glial vascular tissue with squamous epithelium, ependymal lining and papillary structure lined by simple columnarstructure lined by simple columnar epithelium

r/o ectopic adrenal tissue Ectopic adrenal glandFragments of thymus pancreaticFragments of thymus, pancreatic, gastric, small intestine and esophageal tissueFragments of tissue from theFragments of tissue from the respiratory tract

PathologyPathology

Tissue source Final Pathology

r/o bladder tissue Fragments of glial tissue, cartilage with bronchial type glands and gastrointestinal mucosagastrointestinal mucosa

Distal colon Segment of colon, meconium filledFragments of fat with adjacent fibrous tissue

Meninges Fragments of glial tissue with squamous mucosa

Tooth Tooth

Post-op CoursePost op Course

Resolving renal failure, not necessitating dialysisg , g yCr improved to 0.6 before being discharged homeSeen by orthopedics surgery for evaluation of left lower extremity prosthesis

Q ti ?Questions?

Parasitic TwinsParasitic Twinswww.downstatesurgery.org

ContentsContentsEarly Human EmbryologyConcept of twinning IntroductionEtiology and pathogenesisEtiology and pathogenesisDiagnosisCase reportsManagementManagementPrognosis Conclusions

Early Human Embryology (week-1)Early Human Embryology (week 1)

Fertilization Cleavage Blastocyst Formation ImplantantionFertilization Cleavage Blastocyst Formation Implantantion

Early Human EmbryologyEarly Human Embryologywww.downstatesurgery.org

Concept Of TwinningConcept Of Twinning

ZygosityChorionicityAmniocity

Concept of TwinningConcept of Twinning

Concept of Twinningwww.downstatesurgery.org

Concept Of TwinningConcept Of Twinning

TwinsTwinsMonozygotic Dizygotic

Dichorionic MonochorionicDiamniotic Monoamniotic

Monochorionic Conjoined twinsDiamniotic

Symmetrical Asymmetrical

Symmetrical Conjoined Twinswww.downstatesurgery.org

Conjoined TwinsConjoined Twins

Ischiopagus tripus

Asymmetrical Conjoined TwinsAsymmetrical Conjoined Twins

Parasitic Twins Fetus-in-fetuParasitic Twins Fetus in fetu

IntroductionIntroduction

The overall incidence of twinning about 1:87 of all birthsgWide variations in the incidence of dizygotic twinning but constant in monozygotic twinning, 3.5 per 1,000

ipregnanciesMonozygotic twins account for one third of twin births Conjoined twins account for 1% of monozygotic twinsConjoined twins account for 1% of monozygotic twinsParasitic/ heteropagus twins are estimated to account <5% of conjoined twins

IntroductionIntroduction

Parasitic twins occurs when there is a parasite tt h t i d li t d f hi t t f thattachment in a nonduplicated fashion to any part of the

body of the autositeIncidence 1 in 50,000 to 100,000 live births

M. H. Kaufman, The embryology of conjoined twins. Children’s Nervous System, 2004, 20: 508-525 Elizabeth Satter, Sandra Tomita. A case report of an omphalopagus heteropagus (parasitic) twin.

Journal of Pediatric Surgery, volume 43, issue 6, June 2008, pages e37-e39.

Etiology and Pathogenesis

Fission or Fusion?Fission or Fusion?

Fission FusionPrimary hypothesisFailure of a single zygote to divide completely

Union of two initially distinct embryos that took place during earlyto divide completely place during early embryonic periodLogrono et al: DNA study demonstrated dizygositydemonstrated dizygosity in a pair of parasitic twins

M. H. Kaufman, The embryology of conjoined twins. Children’s Nervous System, 2004, 20: 508-525. M. H. Kaufman, The embryology of conjoined twins.

Child ’ N S t 2004 20 508 525y , ,

Children’s Nervous System, 2004, 20: 508-525.

PathogenesisPathogenesis

It is postulated that parasitic twin occur as a result of p pselective ischemic damage in uteroResulting in death and partial resorption of one of the t i d t t i i l t iti t itwins and eventuates in an incomplete parasitic twin attached to a fully developed twinsMechanism maybe similar to that which producesMechanism maybe similar to that which produces acardiac twins

Elizabeth Satter, Sandra Tomita. A case report of an omphalopagus heteropagus (parasitic) twin. Journal of Pediatric Surgery, volume 43, issue 6, June 2008, pages e37-e39

Acardiac TwinsAcardiac Twinswww.downstatesurgery.org

DiagnosisDiagnosis

Prenatalusually by obstetric sonogram

Postnatalcombination of different modalities

Cxray, sonogram, MRI, CT

Case Reportsp

Lumbosacral Parasitic Rachipagus (2006)New Delhi, India

A 2.3 kg full term baby girl was born by C-section to g y g yhealthy parentsNo fetal abnormality suspected prenatallyNo h/o maternal illness or drug intake during pregnancyNo h/o congenital anomalies in familyHas three other healthy siblingsHas three other healthy siblingsAt birth, a 11 x 8 cm mass was attached to the lumbosacral region with bony structure underneathg y

Asymmetric Conjoined Twins: Atypical I hi P it (2007)Ischiopagus Parasite (2007)Chandigarh, India

A 2.7kg full-term baby girl was born to a young healthy woman via normal vaginal deliveryNo h/o maternal illness or drug intake during pregnancyNo h/o maternal illness or drug intake during pregnancyNo h/o congenital anomalies in familyAt birth, a parasitic twin was found attached to her leftAt birth, a parasitic twin was found attached to her left lumbar regionAlso found to have a major exomphalos with a t t d th hi h ti b ltransparent sac, underneath which necrotic bowel was visible

Asymmetric Conjoined Twins: Atypical Ischiopagus Parasite (2007)Ischiopagus Parasite (2007)Chandigarh, India

ManagementManagementwww.downstatesurgery.org

ManagementManagement

Recognition Xray, sonogram, CT, MRI

Management

U lik t i l j i d t i iti t i ftUnlike symmetrical conjoined twins, parasitic twins often do not shared major organsThus surgical separation for parasitic twins is less complicatedcomplicated

Elizabeth Satter, Sandra Tomita. A case report of an omphalopagus heteropagus (parasitic) twin. Journal of Pediatric Surgery, volume 43, issue 6, June 2008, pages e37-e39.

PrognosisPrognosis

The outcome of the autosite after surgical intervention is gusually favorableNo occurrence of recurrence

Nisreen M Khalifa, Doaa W Maximous, Alaa A Abd-elsayed. Department of Pediatric Oncology/ Surgical oncology/ Public Health and Community Medicine, South Egypt Cancer Institute, Asdiut University, Egypt. Fetus in fetu: A case report. http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=2253549, January 10, 2008.p p g g , y ,

ConclusionsConclusions

Parasitic twins are rare, account for less than 5% of ,conjoined twinsTheory of “fission” or “fusion”Occur as a result of selective ischemic damage in uteroPrenatal diagnosis is possible by sonogram Surgery is the mainstay treatment to separate theSurgery is the mainstay treatment to separate the parasite from the autosite, less complicated than conjoined twins separationThe prognosis of the autosite after surgical intervention is excellent

References

1. Phillippe Jeanty, Kay Caldwell, Patricia Dix. Department of Radiology, Vanderbilt University. Fetus-in-fetu. http://www.thefetus.net/page.php?id=290, December 6, 2001.p p g p p , ,

2. Nisreen M Khalifa, Doaa W Maximous, Alaa A Abd-elsayed. Department of Pediatric Oncology/ Surgical oncology/ Public Health and Community Medicine, South Egypt Cancer Institute, Asdiut University Egypt Fetus in fetu: A case reportAsdiut University, Egypt. Fetus in fetu: A case report. http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=2253549, January 10, 2008.

3. 22q11.2 deletion syndrome. http://en wikipedia org/wiki/22q11 2 deletion syndromehttp://en.wikipedia.org/wiki/22q11.2_deletion_syndrome

4. Elizabeth Satter, Sandra Tomita. A case report of an omphalopagus heteropagus (parasitic) twin. Journal of Pediatric Surgery, volume 43, issue 6, June 2008, pages e37-e39.

5. Rajiv Chadha, Prabha Lal, Dharmendra Singh, Akshay Sharma, S. Roy Choudhury, Lumbosacral parasitic rachipagus twin. Journal of Pediatric Surgery, volume 41, 2006, pages e45-e48.

ReferencesReferences

1. J.K. Mahajan, Kumar Devendra, Deb Mainak, and K.L.N Rao, Asymmetric Conjoined Twins: Atypical Ischiopagus Parasite. Journal of Pediatric Surgery. Volume 37, October 2002, page e33.

2. M. H. Kaufman, The embryology of conjoined twins. Children’s Nervous System, 2004, 20: 508-525.

AssessmentAssessment www.downstatesurgery.org

Question 1Question 1

Which of the following is true?g

(a) Fusion is the primary theory for conjoined twins(b) The latter the cleavage, the higher chance of conjoined

embryos in early human embryology( ) Asymmetrical conjoined twins are more common than(c) Asymmetrical conjoined twins are more common than

symmetrical conjoined twins(d) Separation surgery is more complicated in ( ) p g y p

asymmetrical conjoined twins

What is the estimated incidence of parasitic twins?p

(a) 1 in 100 live births(b) 1 in 1000 live births(c) 1 in 10,000 live births(d) 1 in 100,000 live births

What is the postulated mechanism of parasitic twins?p p(a) Maternal malnutrition(b) Maternal trauma(c) Fetal neoplastic growth(d) Twins Reversed Arterial Perfusion syndrome in acardic

twinstwins

Which is the most common type of conjoined twins?yp j(a) Thoragopagus(b) Craniopagus(c) Pygopagus(d) Omphalopagus

What is the recurrence rate for parasitic twins after surgical p gIntervention?(a) None(b) < 2%(c) 10%

%(d) 50%

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